Atrophoderma of Pasini and Pierini (APP) is a rare skin condition of unknown etiology. It is characterized by dermal atrophy with single or multiple hyperpigmented, non-indurated, and slightly depressed plaques on the trunk or limbs; unlike morphea, this condition does not cause induration. In this report, we analyze the case of a patient diagnosed with APP with a 19-year history of progression, aiming to highlight the few cases published in the literature. The patient responded favorably to treatment with mycophenolic acid (MPA). Therapeutic approaches for this condition are controversial due to its long-term progression and low frequency. Currently, there are no published reports on the use of MPA for the treatment of this condition.